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The Brugada syndrome

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Abstract

The Brugada syndrome is a hereditary disease causing sudden cardiac death in apparently healthy individuals with a structurally normal heart. The disease is caused by mutations in the cardiac sodium channel gene SCN5A. Patients with this disease have a peculiar electrocardiogram with elevation of the ST segment in leads V1 to V3, an electrocardiogram that every doctor should recognize. There exist variants of the electrocardiogram with minimal ST segment elevation and even concealed forms that can only be unmasked by the administration of class I antiarrhythmic drugs. When left untreated or when treated with all known antiarrhythmic drugs, patients with Brugada syndrome have a high mortality (approximately 10% per year). The only effective treatment to prevent sudden death is the implantable defibrillator.

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Authors and Affiliations

  1. Cardiovascular Center, OLV Hospital, Moorselbaan 164, 9300, Aalst, Belgium

    Pedro Brugada MD, PhD

  2. Baylor College of Medicine, Houston, TX, USA

    Ramon Brugada MD

  3. Arrhythmia Unit, Hospital Clinic, University of Barcelona, Spain

    Josep Brugada MD, PhD

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  1. Pedro Brugada MD, PhD
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  2. Ramon Brugada MD
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  3. Josep Brugada MD, PhD
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Brugada, P., Brugada, R. & Brugada, J. The Brugada syndrome. Curr Cardiol Rep 2, 507–514 (2000). https://doi.org/10.1007/s11886-000-0035-0

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